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Sweet syndrome is characterized by fever, leukocytosis, and tender, erythematous, well-demarcated papules and plaques, which show dense neutrophilic infiltrates and papillary dermal edema. It may occur in the absence of other diseases but is often associated with malignant conditions and may be induced by various medications. Treatment with systemic glucocorticoids generally is successful but may require prolonged courses to suppress recurrences.
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BACKGROUND: Sweet syndrome is a neutrophilic skin disease that can involve extracutaneous signs. Here we describe a case of aseptic meningitis, a rare potential extracutaneous sign of Sweet syndrome. CASE REPORT: A 42-year-old man was hospitalized for non-pruritic maculoerythematous skin lesions of the legs and back with subsequent myalgia. A histology specimen taken from a skin lesion revealed an acute neutrophilic disease consistent with Sweet syndrome. Marked inflammation and cholestasis were observed. Systemic corticosteroid therapy was given and resulted in good clinical and laboratory response. Two weeks later, in a setting of gradual dosage reduction, the patient was hospitalized for intense headaches associated with meningeal irritation in an inflammatory context. Liver function tests were again abnormal. We concluded on a diagnosis of Sweet syndrome complicated by aseptic meningitis and hepatic sites. Investigation for underlying disease, particularly digestive or hematologic, was negative. A favorable outcome was achieved following administration of higher doses of systemic corticosteroids. DISCUSSION: Aseptic meningitis constitutes an extracutaneous localization of Sweet syndrome. A multidisciplinary approach and exclusion of infectious origin are required in order to institute systemic corticosteroid therapy.
Three patients with coexistent Fanconi syndrome and Sweet syndrome (neutrophilic dermatosis) are presented. These sterile skin lesions responded to systemic corticosteroid therapy in all three cases, and recurred when treatment was discontinued. The association in children of Sweet syndrome with malignancy has previously been recognized; it has not been reported in the premalignant phase of Fanconi anemia. This report expands the differential diagnosis of the neutrophilic dermatoses. Children with Sweet syndrome and anemia should be examined for Fanconi anemia by diepoxybutane cytogenetic studies.
BACKGROUND: Sweet syndrome (acute febrile neutrophilic dermatosis) may occur as a cutaneous paraneoplastic syndrome. This condition has been associated with hematologic malignancies and, to a lesser extent, with solid tumors. METHODS: The authors report two patients with malignancy-associated Sweet syndrome: a 66-year-old man in whom the onset of Sweet syndrome preceded the diagnosis of an adenocarcinoma of unknown primary by 3 months and a 69-year-old woman in whom a workup after the appearance of Sweet syndrome skin lesions revealed an unsuspected recurrent squamous cell carcinoma of the larynx. The authors review the reports of the other 39 patients with solid tumor-associated Sweet syndrome that have been published in the world literature. RESULTS: The most common malignancies were carcinomas of the genitourinary organs (37%), breast (23%), and gastrointestinal tract (17%). Typical clinical features and laboratory findings in these patients included tender erythematous plaques located on the upper extremities (97%); elevated erythrocyte sedimentation rate (95%); anemia (83%); fever (79%); and neutrophilia (60%). The symptoms and lesions of Sweet syndrome resolved after treatment with corticosteroids, potassium iodide, or colchicine. Sweet syndrome preceded the initial diagnosis of cancer or the detection of asymptomatic metastatic, persistent, or recurrent tumor, or a hematologic malignancy (in an individual with a previously diagnosed solid tumor) in 61% of the patients. In the other 39% of patients, diagnosis of Sweet syndrome followed the development of a solid tumor. CONCLUSION: The search for a neoplasm of the genitourinary organs and breast cancer in women and a gastrointestinal tract carcinoma in men should be emphasized in the evaluation for a solid tumor in patients with Sweet syndrome without a prior diagnosis of malignancy.
BACKGROUND: A case of Sweet syndrome mimicking orbital cellulitis is reported. CASE: A 17-year-old girl presented with painful eyelid swelling, limited ocular movement in the right eye, and an increased white cell count. The patient was initially diagnosed as having infectious orbital cellulitis, but her symptoms did not improve with administration of intravenous carbanpenem and cefzon. Systemic examination revealed erythema of the extremities, and blood tests showed elevated C-reactive protein. These findings are consistent with a diagnosis of Sweet syndrome, and the patient was treated with 30 mg of oral prednisone. The eyelid swelling and erythema of the extremities decreased one day after the initiation of corticosteroid therapy. Skin biopsy showed infiltration of neutrophils and histiocytes in the dermis, confirming the diagnosis of Sweet syndrome. CONCLUSION: Sweet syndrome with eyelid and orbital involvement may mimic infectious orbital cellulitis.
BACKGROUND: Sweet syndrome is characterized by painful, erythematous plaques of rapid onset accompanied by fever. Absence of vasculitis is a histologic criterion for diagnosis. However, recent reports suggest that vasculitis should not exclude the diagnosis. We hypothesized that vasculitis can occur in Sweet syndrome and that it represents an epiphenomenon rather than a primary immune-mediated process. DESIGN: Skin biopsy specimens from patients with Sweet syndrome were reviewed to determine the prevalence of vasculitis. The clinicopathologic features of cases with vasculitis were evaluated for statistically significant associations. Specimens with vasculitis underwent immunofluorescence staining. SETTING: University department of dermatology, university hospital, and private practice. PATIENTS: Medical records and biopsy specimens of 21 patients meeting diagnostic criteria for Sweet syndrome were reviewed. INTERVENTIONS: None. RESULTS: The prevalence of vasculitis was 29% (6 of 21 patients). There was a significant association of vasculitis with lesions of longer duration (P =.02). Vascular immunoglobulin and complement could not be demonstrated in cases of Sweet syndrome with vasculitis. CONCLUSIONS: Vasculitis is not a primary, immune-mediated process in Sweet syndrome but occurs secondary to noxious products released from neutrophils. Blood vessels in lesions of longer duration are more likely to develop vasculitis than those of shorter duration because of prolonged exposure to noxious metabolites. Vasculitis does not exclude a diagnosis of Sweet syndrome.
BACKGROUND: Sweet syndrome is an acute neutrophilic dermatosis that occurs with malignant diseases, mainly myeloid hemopathies, in about 20% of cases. When associated with myelodysplasia, Sweet syndrome may be clinically atypical. It can be histologically unusual. Concomitant infiltration of mature neutrophils and immature myeloid cells has been reported, and its significance is still debated. In few patients, lymphocytic infiltrates are the presenting feature of Sweet syndrome with myelodysplasia. OBSERVATIONS: We present 9 male adult patients with chronic Sweet syndrome, all with recurrent eruptions of erythematous and annular plaques that were associated with relapsing polychondritis in 4 of the 9 patients. Results from sequential biopsies showed that infiltrates were initially composed of lymphocytes and that neutrophilic dermal infiltration typical of Sweet syndrome occurred 24 to 96 months later, except in 2 cases. Moreover, atypical mononuclear cells were present on all initial biopsy specimens and strongly reacted to CD68 and myeloperoxidase, indicating a myeloid origin. Myelodysplastic syndrome occurred in all 9 patients, concomitantly with the neutrophilic infiltrate in 4 cases. CONCLUSIONS: Lymphocytic infiltrates with a clinical aspect of Sweet syndrome might represent the initial stage of a cutaneous dysgranulopoiesis syndrome and should lead to the research of atypical myeloid cells in skin infiltrate, blood, and bone marrow for the early detection of an associated myelodysplastic syndrome.
BACKGROUND: Sweet syndrome (acute febrile neutrophilic dermatosis) may arise in association with a variety of underlying systemic diseases. Only 1 case of coccidioidomycosis-associated Sweet syndrome has previously been reported. OBSERVATIONS: We describe 2 patients who developed Sweet syndrome during the onset of acute pulmonary coccidioidomycosis. Systemic antifungal therapy was given in both cases. Respiratory symptoms and skin lesions resolved within 5 weeks. CONCLUSIONS: Sweet syndrome may be a presenting feature of coccidioidomycosis. Recognition of the underlying pulmonary infection is important so that inappropriate treatment with systemic corticosteroids can be avoided.
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BACKGROUND: We describe the first reported case associating coccidioidomycosis and Sweet syndrome, two uncommon diseases in Europe. CASE REPORT: One month after traveling to Mexico, a 47-year-old woman developed a maculo-papulous rash on her trunk, neck and limbs. She also had cough, associated with signs of an upper respiratory tract infection and weight loss. Sweetís syndrome was first diagnosis and confirmed histologically on a skin lesion biopsy. An aspectific pulmonary infiltrate, associated with a left-sided paracardiac opacity was found on chest X-rays and the CT scan. Bronchoalveolar lavage products contained more than 60% lymphocytes. Serology using coccidioidin showed an F-precipitin on agar (IgM) and an IgG-titre of 1:8, leading to the diagnosis of primary Coccidioides immitis infection with a probable lung localization. DISCUSSION: Search for an associated disease should be made in patients with Sweet syndrome. The Sweet syndrome coccidioidomycosis association could be related to TH-1 lymphocyte proliferation.
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Sweet, in 1964, described a skin condition characterized by fever, leukocytosis, tender erythematous plaques, and, histopathologically, a predominantly neutrophilic dermal inflammation. However, other dermatologic conditions can present with similar clinical and histological features. Therefore, diagnostic criteria are important for the correct diagnosis. Use of systemic steroids is the treatment of choice for Sweet syndrome. A number of other medications can be useful at times, such as potassium iodide, dapsone, indomethacin, and colchicine.
Neutrophilic dermatosis (Sweet syndrome) is a rare condition characterized by painful indurated cutaneous plaques infiltrated with mature neutrophils and may be accompanied by fever, granulocytosis, arthritis, and conjunctivitis. It is associated with various malignant and preneoplastic states, the most common being leukemia and myeloproliferative disorders. Its association with solid tumors is infrequent. The case described here represents, to our knowledge, the first report of Sweet syndrome in a patient with osteogenic sarcoma, a primary tumor of bone arising from mesenchymal cells.
Painful erythematous plaques and a pathognomonic, dense, dermal infiltrate of neutrophils characterize Sweet syndrome. Although numerous instances of this disease have been reported in children, only four have had an associated immunodeficiency to date. Sweet syndrome has been traditionally treated with systemic corticosteroids despite an unknown pathogenesis. Prior reports suggest that immunodeficient children with Sweet syndrome may have a persistent course and fail to respond to standard treatment. We describe a child with debilitating Sweet syndrome and a primary immunodeficiency whose symptoms were controlled with intravenous immunoglobulin and dapsone.
OBJECTIVE: To describe a series of 41 patients with fresh lesions of Sweet syndrome in which the histopathologic study demonstrated an inflammatory infiltrate mostly composed of histiocytoid mononuclear cells. DESIGN: Histopathologic, immunohistochemical, and cytogenetic studies of the inflammatory infiltrate in a case series of histiocytoid Sweet syndrome. SETTING: University departments of dermatology and a private laboratory of dermatopathology. METHODS: Conventional histopathologic study as well as immunohistochemical investigations were performed using the alkaline phosphatase antialkaline phosphatase technique with a large panel of antibodies. In some cases, fluorescent in situ hybridization studies were performed to investigate the presence of the bcr/abl gene fusion. RESULTS: Immunohistochemical studies demonstrated that most cells of the infiltrate showed immunoreactivity for CD15, CD43, CD45, CD68, MAC-386, HAM56, and lysozyme, which is consistent with a monocytic-histiocytic immunoprofile. However, intense myeloperoxidase reactivity was detected in most of the cells with histiocytic appearance, which raised the possibility of specific cutaneous involvement by myelogenous leukemia. Nevertheless, cytologic peripheral blood examinations, fluorescent in situ hybridization studies to investigate the bcr/abl gene fusion, and follow-up of the patients, taken all together, ruled out this possibility. CONCLUSIONS: This case series demonstrates that some fresh cutaneous lesions of Sweet syndrome are histopathologically characterized by an infiltrate mostly composed of cells that may be misinterpreted as histiocytes, when in fact they are immature myeloid cells. We named this histopathologic variant histiocytoid Sweet syndrome, which should not be mistaken with leukemia cutis or other inflammatory dermatoses that are histopathologically characterized by histiocytes interstitially arranged between collagen bundles of the dermis.
A case of Sweet syndrome in a patient with an acute myelomonocytic leukemia is reported. It is an unusual case simulating a facial erysipela. This case illustrates the difficulty in differentiating infection from Sweet syndrome in an immunocompromised patient. The negative cultures, ineffective antibiotics, and histopathologic examination results allowed the onset of corticosteroid therapy. The phagocytic function of the neutrophils studied by chemoluminescence was normal in the patient. The physiopathology of Sweet syndrome is discussed, in particular the role of cytokines in the accumulation of granulocytes.